The incidence of congenital heart defects in very low birth weight and extremely low birth weight infants.

BACKGROUND: The incidence of congenital heart defects, reported to be 5-8/1000 in term infants, is not well established in very low birth weight infants. OBJECTIVES: To establish the incidence of congenital heart defects in VLBW infants in the neonatal intensive care unit of our institution. METHODS: A retrospective analysis of the population in the NICU at our institution was performed. VLBW (BW < or = 1500 g) infants born between 2001 and 2006 who survived more than 48 hours were included in the study. Infants with clinical signs of heart disease underwent echocardiography. RESULTS: During the study period 437 VLBW live-born infants met the inclusion criteria. Of these, 281 (64.3%) underwent echocardiography. CHD was detected in 19 infants (4.4%, 95% confidence interval 2.4-5.4%), significantly higher than the incidence of 5-8/1000 in the general population (P<0.0001). In the subgroup of 154 infants with BW < 1000 g there were 10 (6.5%) with CHD. In the subgroup of 283 infants with BW 100-1500 g there were 9 (3.2%, P= 0.19 vs. VLBW) with CHD. CONCLUSIONS: Our observations show an increased incidence of CHD in VLBW neonates, as compared to the general population. Since not all infants underwent echocardiography, and minor cardiac defects may have been missed in our VLBW infants, the true incidence may be higher than reported here.

Cytomegalovirus myocarditis in a healthy infant: Complete recovery after ganciclovir treatment.

OBJECTIVES: To report a case of acute myocarditis caused by cytomegalovirus infection in a 15-month-old immunocompetent infant completely recovered with ganciclovir treatment. DESIGN: Descriptive case report. SETTING: Pediatric intensive care unit in a general hospital. Patient: A 15-month-old healthy girl with acute, severe myocarditis. INTERVENTION: General supportive intensive care and mechanical ventilatory support, iv immunoglobulin, and iv ganciclovir. MEASUREMENTS AND MAIN RESULTS: Intensive supportive care including iv fluids, mechanical ventilatory support, diuretics (furosemide, spironolactone), digoxin, dobutamine, captopril, methylprednisolone, and iv immunoglobulin. Despite clinical stabilization, shortening fraction remained very poor at 17%. Addition of iv ganciclovir resulted in prompt and complete recovery of the cardiac muscle contractility with a shortening fraction of 35% that remained normal during a long follow-up period. CONCLUSIONS: Cytomegalovirus should be considered as a causative agent in acute myocarditis even in the normal, immunocompetent host. In such cases, addition of ganciclovir treatment should be strongly considered.

Outcome of ten years' echocardiographic follow-up in children with Gaucher disease.

The non-neuronopathic form Gaucher disease, the most prevalent lysosomal storage disorder, is marked by tremendous phenotypic heterogeneity; cardio-pulmonary involvement is distinctly rare except in the most severely affected patients. With the advent of enzyme replacement therapy, most symptomatic patients will not suffer from lung disease. However, because of concern about pulmonary hypertension among adult patients exposed to enzyme replacement therapy, echocardiography has been recommended as an early warning system for routine follow-up of all patients, including children. The purpose of this study was to review the results of more than a decade of echocardiographic findings in children followed semi-annually in a large referral clinic in order to ascertain whether echocardiography as an early signal of pulmonary hypertension in children is appropriate. 330 echocardiographic examinations were performed in 71 children (276 patient follow-up years). Only four patients receiving enzyme therapy each had a single abnormal examination that upon repeat examination six months later reverted to within normal limits. There were no abnormal results among the untreated patients. Therefore, we feel comfortable with rescinding our recommendation with regard to routine echocardiographic examinations in children. At the present time we believe that a baseline examination to rule out abnormalities would be sufficient.